- Email: [email protected]
Bilateral Intralobar Pulmonary Sequestration: Therapeutic Implications
CASE REPORTS
Bilateral Intralobar Pulmonarv Seauestration: Therapeutic Implications J
1
F. M. Juettner, M.D., H. H. Pinter, M.D., G. Lammer, M.D., H. Popper, M.D., and G. B. Friehs, M.D.
ABSTRACT Bilateral intralobar pulmonary sequestration within the lower lobes was suggested by computed tomography and confirmed by arteriography in a 27-year-old woman. Although one side was asymptomatic, both sequestrated areas were resected using staged bilateral thoracotomies. Histopathological examination showed massive inflammation in the symptomatic as well as the asymptomatic sequestrum. To our knowlege, this is the sixth reported case of bilateral intralobar pulmonary sequestration and the third for which bilateral resection has been performed.
Intralobar pulmonary sequestration is defined as a congenitally dysplastic area of lung without communication to the bronchial tree that is supplied by an atypical systemic artery usually arising from the aorta. The affected area lies within the pleura of an otherwise normal lobe. Venous drainage runs into either a pulmonary vein or both systemic and pulmonary veins [l].Bilateral occurrence is extremely rare; to our knowledge, only 5 such cases have been documented [2-61. In this article we report a sixth case, which illustrates the need for a bilateral surgical approach even in the absence of clinical symptoms or obvious radiological changes in lung parenchyma on both sides. A 27-year-old woman was seen with left-sided pain in the back. She had noted recurrent attacks of “bronchitis” accompanied by left-sided chest pain during the previous two years. Chest roentgenograms and conventional tomography showed a well-confined opacity in the left costovertebrophrenic angle. Bronchoscopically, a slight ventral displacement of otherwise normal segmental bronchi in both lower lobes was noted. Computed tomographic scan showed a cystic mass within the left posterior basal segment. Discrete infiltrative signs within the right lateral and posterior basal segments were also present. Venous bolus injection suggested a vascular supply to the cystic lesion on the left side arising from the thoracic aorta (Fig 1). Unexpectedly, a second aortopulmonary vessel, supplying the right posterior basal segment, was found (Fig 2). The From the Departments of Thoracic and Hyperbaric Surgery and Radiology and the Institute of Pathology, [Jniversity Medical School, Graz, Austria.
Accepted for publication July 9, 1986. Address reprint requests to Dr.Juettner, Department for Thoracic and Hyperbaric Surgery, University Medical School, A-8036, Graz, Austria.
660 Ann Thorac Surg 43:660-662,June 1987
tentative diagnosis of bilateral pulmonary sequestration was confirmed by aortography: two large-caliber arteries originating in the thoracic aorta at the level of the 10th thoracic vertebra were found to be supplying the left and right lower lobe segments, respectively (Fig 3). Venous drainage was into the inferior pulmonary vein on the right side and into both the hemiazygos and inferior pulmonary veins on the left side. Bilateral staged resection was planned, and the left side was approached first. Through a left-sided thoracotomy, a well-confined, sequestrated area about 10 cm in diameter was found within the posterior basal segment. The outer diameter of the atypical artery was 9 mm; the systemic vein had an hemiazygoslike caliber. The posterior basal segmental artery was extremely hypoplastic. Posterior basal segmentectomy was done. The second operation was delayed at the request of the patient, who remained asymptomatic. Ten months after the first operation, the right-sided thoracotomy was performed. Again, the sequestration was developed within the posterior basal segment, but because of massive collateral inflammation, the sequestrum could not be clearly differentiated from the nonaffected tissue. The atypical vessel had an outer diameter of 7 mm. The posterior basal segmental artery showed a narrowed lumen. The extent of inflammatory changes necessitated resection of all basal segments, and only the superior segment was left intact. Pathological examination of both left-sided and rightsided posterior basal segments revealed severe acute and chronic inflammation within a dysplastic parenchyma showing alveoli and bronchioli hyperdistended by mucus. Pulmonary vessels showed signs of systemic vascularization consistent with the diagnosis of pulmonary sequestration. On the right side, massive inflammatory changes extended into all other basal segments; however, the parenchyma was devoid of either dysplastic changes or signs of atypical vascularization. After an uneventful recovery, the patient returned to active duty and has remained symptomless during an 18-month follow-up.
Comment Intralobar pulmonary sequestration exhibits various morphological features such as cysts, pseudotumors, and atelectases. The clinical appearance varies as well: some patients are seen with severe inflammation, whereas others remain asymptomatic [l]. Even in the absence of symptoms, regions with atypical systemic vascularization should be resected, as there is a likeli-
661
Case Report: Juettner, Pinter, Lammer, et al: Bilateral Intralobar Pulmonary Sequestration
Fig 1 . Computed tomographic scan using a venous bolus injection shows a large-caliber aortopulmonary vessel (arrow)supplying a well-confined cystic area (asterisk)within the left dorsobasal segments.
Fig 3 . Aortogram shuws one large-caliber aortopulmonary vessel supplying the sequestrated area of the left lower lobe (arrow)and a second vessel, arising more centrally, supplying the right lower lobe (as-
terisk). Fig 2 . Computed tomographic scan using a venous bolus injection shows a medium-caliber aortopulmonary vessel (arrow) supplying the right dorsobasal segments.
hood of infection as well as danger of hemorrhage caused by arteriovenous anastomoses. Furthermore, the occurrence of fungal infections, tuberculosis, and even tumors within sequestrated areas has been reported [l, 71. A review of published reports yielded 5 cases of bilateral intralobar pulmonary sequestration, 1 of which was found on postmortem examination [2]. Two of 5 patients had arteriographic evidence of bilateral systemic vascularization to the lower lobes [3, 41, yet routine chest roentgenograms, conventional tomography, and bronchological investigations failed to demonstrate pathological changes on one side, and only the symptomatic side was resected in both patients. In 1 of these 2 patients the anomalous artery to the contralateral side was ligated [4], based on the assumption of mere persistence
of systemic vascularization in an otherwise normal lung. However, this suspicion was not verified by pulmonary arteriography or surgical exploration [8]. Of the 2 patients who underwent bilateral resection, l had clinically and radiologically pathological findings on both sides [6]. In the second patient-similar to our patient-only the right side showed no prominent features other than the atypical vascularization (51. Nevertheless, a bilateral resection was performed prophylactically. Pathological findings in both operative specimens were consistent with intralobar pulmonary sequestration. In our patient, the left-sided sequestration was clearly diagnosed by clinical and radiological findings, whereas we were unable to demonstrate pathological findings in the right lung using routine chest roentgenograms and conventional tomography. Discrete parenchymal changes were seen only on computed tomographic scans, which, by visualizing the feeding arteries, proved to be a valid method of confirming the tentative diagnosis. On the basis of these findings alone, the persistence of systemic arterialization to an otherwise nor-
662 The Annals of Thoracic Surgery Vol 43 No 6 June 1987
ma1 lobe could have been a differential diagnosis for the changes on the right side; however, pathological findings rejected this suggestion. There was massive inflammation in a clearly dysplastic tissue that even affected the surrounding parenchyma. Because the patient had been asymptomatic during the 10-month interval between operations, the changes are unlikely to have occurred in that period. As for the surgical approach, we preferred bilateral thoracotomies to a median sternotomy for reasons of better exposure of both the affected segments and the large atypical vessels, thus minimizing the risk of operative hazards during resection. Any patient with bilateral atypical systemic arterialization of the lung suggestive of pulmonary sequestration should be considered for operation. If routine chest roentgenograms and conventional tomography fail to show parenchymal changes, computed tomography, which is also a valid tool in the noninvasive screening of vascular lesions to the lung, might give further information. Even in the presence of the most discrete radiological signs, pathological examination may reveal a florid inflammatory process.
References 1. Spencer H: Congenital abnormalities of the lung. In Spencer H (ed): Pathology of the Lung. Third ed. Oxford, Pergamon Press, 1977, pp 84-87 2. Gerle RD, Jaretzki A, Ashley CA, Berne AS: Congenital bronchopulmonary-foregut malformation: pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 2781413, 1968 3. Ennis JT, Moule NJ, Woo-Ming M: Intralobar pulmonary sequestration in association with bilateral systemic arterialization of the lungs. Br J Radiol 45:945, 1972 4. Karp W: Bilateral pulmonary sequestration of the lung. AIR 128513, 1977 5. Roe JP, Mack JW, Shirley JH: Bilateral pulmonary sequestrations. J Thorac Cardiovasc Surg 8053, 1980 6. Wimbish KJ, Agha FP, Brady TM: Bilateral pulmonary sequestration: computed tomographic appearance. AJR 140: 689, 1983 7. Juettner FM, Pinter H, Friehs G, Hoefler H: Bronchial carcinoid arising in intralobar bronchopulmonary sequestration with vascular supply from the left gastric artery. J Thorac Cardiovasc Surg 90:25, 1985 8. Ferris EJ, Smith PL, Mirza FH, et al: Intralobar pulmonary sequestration: value of aortography and pulmonary arteriography. Cardiovasc Intervent Radiol 4:17, 1981
Bilateral Intralobar Pulmonarv Seauestration: Therapeutic Implications J
1
F. M. Juettner, M.D., H. H. Pinter, M.D., G. Lammer, M.D., H. Popper, M.D., and G. B. Friehs, M.D.
ABSTRACT Bilateral intralobar pulmonary sequestration within the lower lobes was suggested by computed tomography and confirmed by arteriography in a 27-year-old woman. Although one side was asymptomatic, both sequestrated areas were resected using staged bilateral thoracotomies. Histopathological examination showed massive inflammation in the symptomatic as well as the asymptomatic sequestrum. To our knowlege, this is the sixth reported case of bilateral intralobar pulmonary sequestration and the third for which bilateral resection has been performed.
Intralobar pulmonary sequestration is defined as a congenitally dysplastic area of lung without communication to the bronchial tree that is supplied by an atypical systemic artery usually arising from the aorta. The affected area lies within the pleura of an otherwise normal lobe. Venous drainage runs into either a pulmonary vein or both systemic and pulmonary veins [l].Bilateral occurrence is extremely rare; to our knowledge, only 5 such cases have been documented [2-61. In this article we report a sixth case, which illustrates the need for a bilateral surgical approach even in the absence of clinical symptoms or obvious radiological changes in lung parenchyma on both sides. A 27-year-old woman was seen with left-sided pain in the back. She had noted recurrent attacks of “bronchitis” accompanied by left-sided chest pain during the previous two years. Chest roentgenograms and conventional tomography showed a well-confined opacity in the left costovertebrophrenic angle. Bronchoscopically, a slight ventral displacement of otherwise normal segmental bronchi in both lower lobes was noted. Computed tomographic scan showed a cystic mass within the left posterior basal segment. Discrete infiltrative signs within the right lateral and posterior basal segments were also present. Venous bolus injection suggested a vascular supply to the cystic lesion on the left side arising from the thoracic aorta (Fig 1). Unexpectedly, a second aortopulmonary vessel, supplying the right posterior basal segment, was found (Fig 2). The From the Departments of Thoracic and Hyperbaric Surgery and Radiology and the Institute of Pathology, [Jniversity Medical School, Graz, Austria.
Accepted for publication July 9, 1986. Address reprint requests to Dr.Juettner, Department for Thoracic and Hyperbaric Surgery, University Medical School, A-8036, Graz, Austria.
660 Ann Thorac Surg 43:660-662,June 1987
tentative diagnosis of bilateral pulmonary sequestration was confirmed by aortography: two large-caliber arteries originating in the thoracic aorta at the level of the 10th thoracic vertebra were found to be supplying the left and right lower lobe segments, respectively (Fig 3). Venous drainage was into the inferior pulmonary vein on the right side and into both the hemiazygos and inferior pulmonary veins on the left side. Bilateral staged resection was planned, and the left side was approached first. Through a left-sided thoracotomy, a well-confined, sequestrated area about 10 cm in diameter was found within the posterior basal segment. The outer diameter of the atypical artery was 9 mm; the systemic vein had an hemiazygoslike caliber. The posterior basal segmental artery was extremely hypoplastic. Posterior basal segmentectomy was done. The second operation was delayed at the request of the patient, who remained asymptomatic. Ten months after the first operation, the right-sided thoracotomy was performed. Again, the sequestration was developed within the posterior basal segment, but because of massive collateral inflammation, the sequestrum could not be clearly differentiated from the nonaffected tissue. The atypical vessel had an outer diameter of 7 mm. The posterior basal segmental artery showed a narrowed lumen. The extent of inflammatory changes necessitated resection of all basal segments, and only the superior segment was left intact. Pathological examination of both left-sided and rightsided posterior basal segments revealed severe acute and chronic inflammation within a dysplastic parenchyma showing alveoli and bronchioli hyperdistended by mucus. Pulmonary vessels showed signs of systemic vascularization consistent with the diagnosis of pulmonary sequestration. On the right side, massive inflammatory changes extended into all other basal segments; however, the parenchyma was devoid of either dysplastic changes or signs of atypical vascularization. After an uneventful recovery, the patient returned to active duty and has remained symptomless during an 18-month follow-up.
Comment Intralobar pulmonary sequestration exhibits various morphological features such as cysts, pseudotumors, and atelectases. The clinical appearance varies as well: some patients are seen with severe inflammation, whereas others remain asymptomatic [l]. Even in the absence of symptoms, regions with atypical systemic vascularization should be resected, as there is a likeli-
661
Case Report: Juettner, Pinter, Lammer, et al: Bilateral Intralobar Pulmonary Sequestration
Fig 1 . Computed tomographic scan using a venous bolus injection shows a large-caliber aortopulmonary vessel (arrow)supplying a well-confined cystic area (asterisk)within the left dorsobasal segments.
Fig 3 . Aortogram shuws one large-caliber aortopulmonary vessel supplying the sequestrated area of the left lower lobe (arrow)and a second vessel, arising more centrally, supplying the right lower lobe (as-
terisk). Fig 2 . Computed tomographic scan using a venous bolus injection shows a medium-caliber aortopulmonary vessel (arrow) supplying the right dorsobasal segments.
hood of infection as well as danger of hemorrhage caused by arteriovenous anastomoses. Furthermore, the occurrence of fungal infections, tuberculosis, and even tumors within sequestrated areas has been reported [l, 71. A review of published reports yielded 5 cases of bilateral intralobar pulmonary sequestration, 1 of which was found on postmortem examination [2]. Two of 5 patients had arteriographic evidence of bilateral systemic vascularization to the lower lobes [3, 41, yet routine chest roentgenograms, conventional tomography, and bronchological investigations failed to demonstrate pathological changes on one side, and only the symptomatic side was resected in both patients. In 1 of these 2 patients the anomalous artery to the contralateral side was ligated [4], based on the assumption of mere persistence
of systemic vascularization in an otherwise normal lung. However, this suspicion was not verified by pulmonary arteriography or surgical exploration [8]. Of the 2 patients who underwent bilateral resection, l had clinically and radiologically pathological findings on both sides [6]. In the second patient-similar to our patient-only the right side showed no prominent features other than the atypical vascularization (51. Nevertheless, a bilateral resection was performed prophylactically. Pathological findings in both operative specimens were consistent with intralobar pulmonary sequestration. In our patient, the left-sided sequestration was clearly diagnosed by clinical and radiological findings, whereas we were unable to demonstrate pathological findings in the right lung using routine chest roentgenograms and conventional tomography. Discrete parenchymal changes were seen only on computed tomographic scans, which, by visualizing the feeding arteries, proved to be a valid method of confirming the tentative diagnosis. On the basis of these findings alone, the persistence of systemic arterialization to an otherwise nor-
662 The Annals of Thoracic Surgery Vol 43 No 6 June 1987
ma1 lobe could have been a differential diagnosis for the changes on the right side; however, pathological findings rejected this suggestion. There was massive inflammation in a clearly dysplastic tissue that even affected the surrounding parenchyma. Because the patient had been asymptomatic during the 10-month interval between operations, the changes are unlikely to have occurred in that period. As for the surgical approach, we preferred bilateral thoracotomies to a median sternotomy for reasons of better exposure of both the affected segments and the large atypical vessels, thus minimizing the risk of operative hazards during resection. Any patient with bilateral atypical systemic arterialization of the lung suggestive of pulmonary sequestration should be considered for operation. If routine chest roentgenograms and conventional tomography fail to show parenchymal changes, computed tomography, which is also a valid tool in the noninvasive screening of vascular lesions to the lung, might give further information. Even in the presence of the most discrete radiological signs, pathological examination may reveal a florid inflammatory process.
References 1. Spencer H: Congenital abnormalities of the lung. In Spencer H (ed): Pathology of the Lung. Third ed. Oxford, Pergamon Press, 1977, pp 84-87 2. Gerle RD, Jaretzki A, Ashley CA, Berne AS: Congenital bronchopulmonary-foregut malformation: pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 2781413, 1968 3. Ennis JT, Moule NJ, Woo-Ming M: Intralobar pulmonary sequestration in association with bilateral systemic arterialization of the lungs. Br J Radiol 45:945, 1972 4. Karp W: Bilateral pulmonary sequestration of the lung. AIR 128513, 1977 5. Roe JP, Mack JW, Shirley JH: Bilateral pulmonary sequestrations. J Thorac Cardiovasc Surg 8053, 1980 6. Wimbish KJ, Agha FP, Brady TM: Bilateral pulmonary sequestration: computed tomographic appearance. AJR 140: 689, 1983 7. Juettner FM, Pinter H, Friehs G, Hoefler H: Bronchial carcinoid arising in intralobar bronchopulmonary sequestration with vascular supply from the left gastric artery. J Thorac Cardiovasc Surg 90:25, 1985 8. Ferris EJ, Smith PL, Mirza FH, et al: Intralobar pulmonary sequestration: value of aortography and pulmonary arteriography. Cardiovasc Intervent Radiol 4:17, 1981